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多发性内分泌腺瘤病 1 型表现为耐药性癫痫和多发性神经病——病例报告。

Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy--a case report.

机构信息

Department of Neurology, Neurology Ward, Universidade de São Paulo, Brazil.

出版信息

J Neurol Sci. 2012 Apr 15;315(1-2):172-5. doi: 10.1016/j.jns.2011.11.015. Epub 2011 Dec 2.

Abstract

Hypoglycemia is a well recognized cause of acute symptomatic seizures. The fact that hypoglycemia can cause peripheral neuropathy is less appreciated. We describe a case of insulinoma associated peripheral neuropathy. A 17 year-old previously healthy man was referred for investigation of refractory epilepsy. A history of recurrent seizures, slowly progressive weakness of his feet and hands, and weight gain was obtained. Physical examination showed signs of a chronic sensory-motor polyneuropathy. He was diagnosed with insulinoma and primary hyperparathyroidism, characterizing multiple endocrine neoplasia, type 1 syndrome. Cases of insulinoma associated peripheral neuropathy are very rare. The more characteristic clinical picture appears to be distal weakness, worse in the intrinsic hand and feet muscles, and no or mild sensory signs. Peripheral nervous system symptoms may not completely resolve, despite removal of the cause of hyperinsulinism/hypoglycemia and full reversion of central nervous system symptoms. Mechanisms underlying hypoglycemic neuropathy are still poorly understood.

摘要

低血糖是急性症状性癫痫发作的一个众所周知的原因。低血糖可引起周围神经病这一事实则较少被人认识。我们描述了一例与胰岛素瘤相关的周围神经病。一名 17 岁的既往健康男性因难治性癫痫而被转来就诊。他有反复发作癫痫、逐渐出现手脚无力和体重增加的病史。体格检查显示出慢性感觉运动性多发性神经病的征象。他被诊断为胰岛素瘤和甲状旁腺功能亢进症,这是多发性内分泌肿瘤 1 型综合征的特征。与胰岛素瘤相关的周围神经病的病例非常罕见。更具特征性的临床表现为远端无力,手部和脚部内在肌肉更为严重,无或轻度感觉征象。尽管消除了高胰岛素血症/低血糖的原因,并且中枢神经系统症状完全逆转,周围神经系统症状仍可能无法完全缓解。低血糖性神经病的发病机制仍知之甚少。

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